ABSTRACT. Background: Gorlin-Goltz syndrome, or basal cell nevus syndrome (BCNS), is a rare autosomal dominant disorder caused by mutations in the Patched (PTCH) gene. BCNS affects about 1:60,000 people and de novo mutations represent approximately 20 % to 30 % of all cases. Purpose: To describe the diagnostic process and management of a rare case of a female patient with BCNS and squamous cell carcinoma (SCC) in upper lip. Case description: The patient was a 58-year-old woman who attended the dental office seeking aesthetic and functional treatment. An asymptomatic, delimited SCC with red erosions and scab in left upper lip was diagnosed (confirmed through immunohistochemistry). Associated lymph adenopathy was not observed. The patient also presented palmoplantar pits and multiple basal cell carcinomas (BCC) in back and left-hand back (and similar family history). X-rays did not show mandible keratocysts often associated to the Gorlin-Goltz syndrome. Conclusions: The patient was diagnosed with BCNS because she met two major (two or more BCCs and palmoplantar pits) and two minor criteria (lamellar calcification of brain sickle and history of ovary fibromas). Dental treatment was planned and the patient was referred to another institution to address her systemic condition.